Frontal and Vascular Dementia

Question 1

Of the dementias, how common is alzheimer’s

Answer 1

50-70% of dementias

Question 2

Of the dementias, how common is vascular dementia?

Answer 2

20-30%

Question 3

Of the dementias, how common is fronto-temporal dementia

Answer 3

5-10%

Question 4

What is the diagnostic criteria for fronto-temporal dementia?

Answer 4

• meet criteria for major and mild NCD
• Insidious onset/gradual progression
• behavioural variant or language variant
• sparing of learning and memory, and perceptual-motor func
• not better explained by another NCD

can also be diagnosed if either there is a famly history of causative mutation, or disproportionate frontal orr temporal lobe involvement.

Question 5

What are the variants of FTD

Answer 5

• behavioural-variant FTD
• Semantic dementia
• progressive nonfluent aphasia
• AD

Question 6

briefly describe bvFTD

Answer 6

behavioural-variant FTD

changes in social conduct, personality and motivation, lack of empathy and insight.

Question 7

briefly describe semantic dementia

Answer 7

loss of semantic knowledge, impaired recognition of objects, faces and emotions

Question 8

briefly describe progressive nonfluent aphasia

Answer 8

effortful, laboured, distorted speech, reduced word output, relatively intact comprehension

like broca’s

Question 9

describe alzheimer’s disease

Answer 9

impaired memory, processing speed, attention.

preserved social graces

Question 10

what is the most common variant of FTD

Answer 10

bvFTNCD

Question 11

what happens if someone with FTD has temporal lobe dysfunction

Answer 11

Language Variant Progressive Non-fluent Aphasia (PNFA) - 20% of ftd cases

receptive language deficits
expressive language deficits

Eventually, many patients with PNFA develop severe Parkinsonian symptoms overlapping with Progressive Supranuclear Palsy (PSP) and Corticobasal degeneration (CBD) – inability to move eyes side-to- side, muscle rigidity in arms and legs, falls, and weakness of muscles around the throat.

Question 12

what is onset like in semantic dementia?

Answer 12

Left Hemispheric onset – loss of meaning for words, decline in reading and spelling, decline in people’s names; Memory not affected until later; intact orientation to place and time, intact muscle control

Right Hemispheric onset – trouble recalling faces of friends and familiar people; deficits understanding emotions of others; loss of empathy

Eventually both hemispheres will become dysfunctional

SD patients eventually develop behavioural problems – disinhibition, apathy, diminished insight

Question 13

Describe someone with FT NCD with motor neurone disease

Answer 13

15% of people with FT NCD develop MND (happens the other way round too)

 MND affects motor nerve cells in spinal cord, brain stem, and cerebral cortex
 Motor symptoms = tremors, jerks (chorea or myoclonus), excessive startle response, seizures
 More frequently found in patients with bvFTNCD
 Rare in SD or PNFA
 Most common MND is amyotrophic lateral sclerosis (ALS). Often patients with ALS have behavioural or cognitive problems similar to those seen in FTNCD.
 MND Symptoms: slurring of speech, difficulty swallowing, choking, limb weakness, or muscle wasting.
 Often a family history of the disease

Question 14

neuropsychiatric features of FT NCD?

Answer 14

 Inattention
 Low Motivation, Apathy or inertia
 Poor Insight
 behavioural disinhibition
 Aggression
 Impulsivity
 Sexual Impropriety
 Loss of sympathy or empathy
 Perseverative, stereotyped or compulsive behaviour  Hyperorality and dietary changes

Question 15

Diagnostic criteria for vascular NCD?

Answer 15

Onset of the cognitive deficits is temporally related to one or more vascular events
OR
Evidence for decline is prominent in complex attention (including processing speed) and frontal-executive function

evidence of cerebrovascular disease from history

not better explained by any other NCD

Question 16

Describe Vascular dementia

Answer 16

2nd most common dementia cause in elderly

highest 5 year mortality rate 61%

potentially preventable, or slowable - eg. cholesterol lowering therapy, tight BP and BG control…

usually need a stroke for this, it’s a step-wise deterioration, where with each stroke, you get a decline

Question 17

Types of vascular NCD?

Answer 17

1. CADASIL (Cerebral autosomal dominant arteriopathy with leukoencephyalopathy) gene mutation Notch 3
2. Multi-Infarct Dementia
3. Subcortical Ischemic Vascular Dementia (SIVD)

Question 18

What is the ischaemic penumbra

Answer 18

this is the rim of the ischaemic tissue, where there is mild ischaemia. this is because it is supplied by collateral arteries.

targetting this area is most effective for treatment

Question 19

What type of stroke is most common in vascular NCD

Answer 19

ischaemic - 83%

can have artherosclerotic, embolism, and lacunar

Question 20

What kind of haemorrhage can cause a vascular NCD?

Answer 20

intracerebral or subarachnoid.

Question 21

Decribe the neuropsychology in Vascular NCD

Answer 21

Depends on type and location (s) of underlying disease, e.g. large vessels in cortical regions cf multiple subcortical.

Cortical infarcts may lead to aphasia, apraxia, and seizures, occurring in close temporal relation to onset of dementia. With focal neurological signs and a classic stepwise progression of cognitive impairment

Subcortical lesions are associated with executive dysfunction, gait disturbance, urinary incontinence, Parkinsonism and bradyphrenia. More insidious course. Often see relatively preserved memory with impaired speed, attention, and executive function.

Depression occurs in up to 20 % of patients with VaD, particularly with frontal white matter involvement

Question 22

What are some neuropsychiatric symptoms in vascular NCD

Answer 22

 Personality change
 Apathy / Dependent behaviours / lack of insight
 Impaired social communication with family and friends
 Mistrust
 Repetitive fixated behaviours
 Neglect of hygiene and appearance
 Guilt or shame
 Generalized Anxiety
 Frequently Depressed
 Agitation /Anger /Disrespectful
 Increased risk of self harm – impulsivity, dangerous risk- taking
 When severe, may have delusions, hallucinations, delirium

Question 23

Of the dementias, which have gradual and which have acute onset?

Answer 23

mNCD AD - gradual, progressive decline, ADL deficits, aphasia, apraxia etc.

vascular - acute onset, stepwise decline, vascular risks, frontal deficits.

Question 24

what is Corticobasal syndrome

Answer 24

a rare progressive neurodegenerative disease involving the cortex and the basal ganglia.